February 3, 2012 mcfadyena

Observations from the trial

Most people who know Trey and know about the trial, want to know what changes I’ve seen in Trey. He’s now had three doses of intrathecal Elarpase. He gets his fourth dose this coming Tuesday, January 7th.
I posted some of the initial changes I saw, back in November 2011: an increased ability to get along and play with his siblings, more attempts at saying three syllable words, an increase in focus, attention and perseverance.
Trey still has trouble being inside for lengths of time. He gets ‘antsy’ and spends a lot of time whacking people’s bums or making loud sounds with instruments, usually drum sticks and shakers, when he needs to get outside (fortunately, once he’s out and about, these behaviours disappear, so for us it’s mostly about how to work with them in the house). Melissa Hogan’s blog, Newly Diagnosed Families Handling the Stares and Comments, helped me with this: http://www.savingcase.com/index.php/2011/12/29/newly-diagnosed-families-handling-the-stares-and-comments/
“Cognitively affected MPS children often have limited ability to control their hyperactivity, climbing, jumping, aggression, repetitive behaviors, fits, unique activities like spanking, grabbing, or touching others, etc. The key is to constantly be testing what they seem to be able to control. Do they understand consequences? Do they understand timeouts? Do they repeat a behavior only minutes after you’ve clearly told them to stop? Is the behavior akin to breathing for them, they need to do it so much? Depending on the answers to those questions, certain behaviors simply need to be managed instead of disciplined or prevented, although we can often try to prevent the triggers of certain behaviors.”
Right now at least, the loud sounds and spanking, seem to be a ‘need’ for Trey (I liked Melissa’s differentiation between managing and disciplining behaviours). The neat part now is, we can ask Trey to do these activities in ways that don’t bother people; by asking permission, by finding another way to fill this need (ie. pillow fights as opposed to spanking), by playing loud sounds in a different room or finding different instruments that are quieter, and he ‘gets’ it. The behaviours haven’t stopped, but there is a change in communication, understanding and ability.
Melissa (I refer to her a lot in my blog because she spends a lot of time thinking and contemplating and as a result has a lot of brilliant insight) described her observation that the difference between kids who are cognitively impacted and those who are not, is that kids who are cognitively okay can have engaging and interactive conversations. With kids who are cognitively impacted, it is like you and they are having ‘passing conversations.’ It’s like you’re going in opposite directions on the highway and are passing each other. There’s a connection that is missed. Her description clicked- it was like a light went on for me. With Trey, you’d ask him one question and he’d answer a different question. Or he’d ask you the same question (ie. what colour is Haley’s house?) repeatedly.
This, the ‘passing conversations’, has lessened. Trey still sometimes needs help answering the question you ‘really’ asked, but he more often answers the question you asked. He still sometimes repeats questions, but he now asks many more questions and repeats less often. This also factors into language changes we’ve noticed. His sentence length and vocabulary have increased. He’s even said a few complete sentences with plurals, the less important ‘the’ and ‘a’ type words, and all.
He is also more affectionate with me, which I LOVE. He’s always been a daddy’s boy, but in the past year or two, he’s wanted even less affection from me. He’d push me away if I went in for a hug, ‘put up with’ but resisted bedtime kisses etc. In the past couple weeks, he’s asked for hugs and snuggles!
Before this trial, it was getting increasingly harder to interact with Trey, especially in a group, because he would lose interest so quickly and ‘check out.’ I wouldn’t say the changes I’ve noticed are drastic, but they are a big deal. It is easier to engage and be in relationship with Trey now.


We want to hear from you! Send us your story so we can share it with the world. Every person and family battling Hunter Syndrome has a unique story to tell - one of bravery, resilience, and perseverance. We'll share all stories online here and on our social media feeds!